Head and Neck Pathology

, Volume 11, Issue 4, pp 525–530 | Cite as

Pseudomyogenic Hemangioendothelioma: A Vascular Tumor Previously Undescribed in the Oral Cavity

  • Yeshwant B. RawalEmail author
  • Kenneth M. Anderson
  • Thomas B. Dodson
Case Report


The pseudomyogenic hemangioendothelioma (PMH) is a low-grade malignant vascular neoplasm of different tissue planes including skin and soft tissue. Primary tumors in the skeletal muscle and bone have also been diagnosed. The PMH was introduced into the WHO classification of tumors of soft tissue and bone in 2013. This is the first description of oral involvement. A 21-year-old female presented with a 2-month old swelling of her gingiva. The swelling appeared red in color and was soft in consistency. A clinical diagnosis of a pyogenic granuloma was made and an incisional biopsy was submitted for histopathological evaluation. The lesion consisted of a proliferation of spindle and epithelioid looking cells. Cells were arranged in loose fascicles and sheets. Rhabdomyoblast-like cells were also seen. No mitotic figures were present. Lesional cells were reactive to cytokeratin AE1/AE3 and CD31. Lesional cell reactivity to S100 protein, HMB 45, SMA, Desmin and CD34 was negative. Following the diagnosis, a wide excision for clear margins was performed. No recurrence has been reported 2 years since the removal. The PMH is a cutaneous tumor that behaves in an indolent fashion. This is the first report of oral involvement by this neoplasm. Recognition of its histopathological features and immunohistochemical reactivity will prevent misadventures in the diagnosis of oral lesions.


Pseudomyogenic Hemangioendothelioma Oral Mucosal Cytokeratin CD31 Epithelioid sarcoma Rhabdomyoblast 


Compliance with Ethical Standards

Conflict of interest

The authors declare that they have no conflict of interest associated with this report.

Ethical Approval

This article does not contain any studies with human participants or animals performed by any of the authors. UTHSC IRB 16-04954-NHSR.


  1. 1.
    Mirra JM, Kessler S, Bhuta S, Eckardt J. The fibroma-like variant of epithelioid sarcoma. A fibrohistiocytic/myoid cell lesion often confused with benign and malignant spindle cell tumors. Cancer. 1992;69(6):1382–95.CrossRefPubMedGoogle Scholar
  2. 2.
    Billings SD, Folpe AL, Weiss SW. Epithelioid sarcoma-like hemangioendothelioma. Am J Surg Pathol. 2003;27(1):48–57.CrossRefPubMedGoogle Scholar
  3. 3.
    Hornick JL, Fletcher CD. Pseudomyogenic (“fibroma-like”) variant of epithelioid sarcoma: a distinctive tumor type with a propensity for multifocality in a single limb but surprisingly indolent behavior. Mod Pathol. 2008;21(suppl 1):13.Google Scholar
  4. 4.
    Hornick JL, Fletcher CD. Pseudomyogenic hemangioendothelioma: a distinctive, often multicentric tumor with indolent behavior. Am J Surg Pathol. 2011;35:190.CrossRefPubMedGoogle Scholar
  5. 5.
    Trombetta D, Magnusson L, von Steyern FV, Hornick JL, Fletcher CD, Mertens F. Translocation (7;19)(q22;q13)—a recurrent chromosome aberration in pseudomyogenic hemangioendothelioma? Cancer Genet. 2011;204(4):211–5.CrossRefPubMedGoogle Scholar
  6. 6.
    Fletcher CDM, Bridge JA, Hogendoorn PCW, Mertens F editors. World Health Organization classification of tumours of soft tissue and bone, 4th edn. Lyon: IARC Press, 2013.Google Scholar
  7. 7.
    Walther C, Tayebwa J, Lilljebjörn H, Magnusson L, Nilsson J, von Steyern FV, et al. A novel SERPINE1-FOSB fusion gene results in transcriptional up-regulation of FOSB in pseudomyogenic haemangioendothelioma. J Pathol. 2014;232(5):534–40.CrossRefPubMedGoogle Scholar
  8. 8.
    Viswanathan S, Rahman K, Pallavi S, Sachin J, Patil A, Chaturvedi P, D’Cruz A, Agarwal J, Kane SV. Sarcomatoid (spindle cell) carcinoma of the head and neck mucosal region: a clinicopathologic review of 103 cases from a tertiary referral cancer centre. Head Neck Pathol. 2010;4(4):265–75.CrossRefPubMedPubMedCentralGoogle Scholar
  9. 9.
    Stefanaki C, Stefanaki K, Chardalias L, Soura E, Stratigos A. Differential diagnosis of Spitzoid melanocytic neoplasms. J Eur Acad Dermatol Venereol. 2016;30(8):1269–77.CrossRefPubMedGoogle Scholar
  10. 10.
    Ohsie SJ, Sarantopoulos GP, Cochran AJ, Binder SW. Immunohistochemical characteristics of melanoma. J Cutan Pathol. 2008;35(5):433–44.CrossRefPubMedGoogle Scholar
  11. 11.
    Volpicelli ER, Fletcher CD. Desmin and CD34 positivity in cellular fibrous histiocytoma: an immunohistochemical analysis of 100 cases. J Cutan Pathol. 2012;39(8):747–52.CrossRefPubMedGoogle Scholar
  12. 12.
    Foss RD, Ellis GL. Myofibromas and myofibromatosis of the oral region: A clinicopathologic analysis of 79 cases. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2000;89(1):57–65.CrossRefPubMedGoogle Scholar
  13. 13.
    Erickson-Johnson MR, Chou MM, Evers BR, Roth CW, Seys AR, Jin L, et al. Nodular fasciitis: a novel model of transient neoplasia induced by MYH9-USP6 gene fusion. Lab Invest. 2011;91(10):1427–33.CrossRefPubMedGoogle Scholar
  14. 14.
    Perez-Montiel MD, Plaza JA, Dominguez-Malagon H, Suster S. Differential expression of smooth muscle myosin, smooth muscle actin, h-caldesmon, and calponin in the diagnosis of myofibroblastic and smooth muscle lesions of skin and soft tissue. Am J Dermatopathol. 2006;28(2):105–11.CrossRefPubMedGoogle Scholar
  15. 15.
    Iwata J, Fletcher CD. Immunohistochemical detection of cytokeratin and epithelial membrane antigen in leiomyosarcoma: a systematic study of 100 cases. Pathol Int. 2000;50(1):7–14.CrossRefPubMedGoogle Scholar
  16. 16.
    Eley KA, Alroyayamina S, Golding SJ, Tiam RN, Watt-Smith SR. Angioleiomyoma of the hard palate: report of a case and review of the literature and magnetic resonance imaging findings of this rare entity. Oral Surg Oral Med Oral Pathol Oral Radiol. 2012;114(2):e45–9.CrossRefPubMedGoogle Scholar
  17. 17.
    Stock N, Chibon F, Binh MB, Terrier P, Michels JJ, Valo I, et al. Adult-type rhabdomyosarcoma: analysis of 57 cases with clinicopathologic description, identification of 3 morphologic patterns and prognosis. Am J Surg Pathol. 2009;33(12):1850–9.CrossRefPubMedGoogle Scholar
  18. 18.
    Thway K, Fisher C. Synovial sarcoma: defining features and diagnostic evolution. Ann Diagn Pathol. 2014;18(6):369–80.CrossRefPubMedGoogle Scholar
  19. 19.
    Miettinen M, Fanburg-Smith JC, Virolainen M, Shmookler BM, Fetsch JF. Epithelioid sarcoma: an immunohistochemical analysis of 112 classical and variant cases and a discussion of the differential diagnosis. Hum Pathol. 1999;30(8):934–42.CrossRefPubMedGoogle Scholar

Copyright information

© Springer Science+Business Media New York 2016

Authors and Affiliations

  • Yeshwant B. Rawal
    • 1
    Email author
  • Kenneth M. Anderson
    • 2
  • Thomas B. Dodson
    • 3
  1. 1.Department of Oral and Maxillofacial Surgery, School of DentistryUniversity of Washington, B-204 Magnuson Health Sciences CenterSeattleUSA
  2. 2.Diagnostic Sciences and Oral Medicine, College of DentistryUniversity of Tennessee Health Science CenterMemphisUSA
  3. 3.Department of Oral and Maxillofacial SurgeryUniversity of Washington School of DentistrySeattleUSA

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